Introduction: Neurofibromatosis type-1 (NF-1) is one of the most common genetic diseases following an autosomal dominant inheritance pattern. Maternal and fetal complications have been reported. Purpose: To present a very interesting and rare case report regarding neurofibromatosis – 1 (NF-1) in pregnancy and to create a complete review concerning this genetic disease. Materials and Methods: Articles were identified through electronic databases; no date or language restrictions were placed; relevant citations were hand searched. The search was conducted using the following terms: neurofibromatosis, neurofibromatosis type-1, pregnancy. Case presentation: We present a case of a 36-year-old nulliparous pregnant woman affected by NF-1. She presented with café-au-lait spots and cutaneous/subcutaneous neurofibromas, progressively increasing in size and number. The unique obstetric complication was placenta previa, diagnosed in the second trimester. A caesarean section was performed on the 36th week. A healthy male neonate unaffected by NF-1 was born. Both post-operative period and puerperium were uneventful. Conclusions: This case report highlights the fact that a normal pregnancy outcome can occur in pregnant women with NF-1 and proper counselling should be in place so that informed decisions can be made by future parents.